Risk Factors of Long-Term Sequelae After Transcatheter Closure of Perimembranous Ventricular Septal Defect in Young Children.

BACKGROUND: Complications arising from transcatheter closure of perimembranous ventricular septal defects (pmVSD) in children, such as residual shunts and aortic regurgitation (AR), have been observed. However, the associated risk factors remain unclear. This study identified risk factors linked with residual shunts and AR following transcatheter closure of pmVSD in children aged 2-12 years.Methods and Results: The medical records of 63 children with pmVSD and a pulmonary-to-systemic blood flow ratio <2.0 who underwent transcatheter closure between 2011 and 2018 were analyzed with a minimum 3-year follow-up. The success rate of transcatheter closure was 98.4%, with no emergency surgery, permanent high-degree atrioventricular block, or mortality. Defects ≥4.5 mm had significantly higher odds of persistent residual shunt (odds ratio [OR] 6.85; P=0.03). The use of an oversize device (≥1.5 mm) showed a trend towards reducing residual shunts (OR 0.23; P=0.06). Age <4 years (OR 27.38; 95% confidence interval [CI] 2.33-321.68) and perimembranous outlet-type VSD (OR 11.94, 95% CI 1.10-129.81) were independent risk factors for AR progression after closure. CONCLUSIONS: Careful attention is crucial for pmVSDs ≥4.5 mm to prevent persistent residual shunts in transcatheter closure. Assessing AR risk, particularly in children aged <4 years, is essential while considering the benefits of pmVSD closure.

Safety and efficacy of transcatheter closure of outlet-type ventricular septal defects in children and adults with Amplatzer Duct Occluder II.

BACKGROUND/PURPOSE: Outlet-type VSD is frequently associated with aortic valve prolapse that surgery is frequently required. The literature regarding outcomes of transcatheter closure of outlet-type VSDs is scant. This study was conducted to know the safety and efficacy of transcatheter closure of outlet-type ventricular septal defects (VSDs) with Amplatzer Duct Occluder II (ADO II). METHODS: Medical records of patients underwent attempted transcatheter closure of outlet-type VSD with ADO II between October 2013 and August 2019 were retrospectively reviewed. RESULTS: Among 49 patients, transcatheter closure was successful in 45 (91.8%; 33 males and 12 females; mean [± standard deviation] age and body weight: 15.8 (±17.7) years and 36.6 (±23.3) kg, respectively). The median VSD diameter was 4.0 mm (range: 1.2-6.0 mm). Device closure failed in four because the sheath could not be advanced through a prograde or retrograde route in one patient, occluder embolization in the two patients, and failed right ventricular disc anchoring in one patient. After a mean follow-up of 22.7 months (range: 0.3-51.1 months), only nine (20.0%) patients had increased severity in aortic regurgitation (AR) on the echocardiography. Preprocedural AR decreased in severity or even disappeared in 11 (24.4%) patients. No heart block or device failure occurred during follow-up. A trivial-to-small residual shunt was detected in 19 patients (42.2%) in the most recent echocardiography. CONCLUSION: Transcatheter closure of outlet-type VSDs with ADO II is feasible. Although no significant aggravation of AR was observed in the short-to-mid-term follow-up, long-term follow-up is mandatory.

Midterm follow-up results of transcatheter treatment in patients with unroofed coronary sinus.

OBJECTIVE: We present the midterm results of transcatheter treatment of unroofed coronary sinus (CS) using the Amplatzer septal occluder. BACKGROUND: The unroofed CS is a rare atrial septal defect communicating the left atrium and CS. Surgery has been the mainstay of treatment. METHODS: In a 4.5-year period, nine patients (five males) with ages ranging from 26 to 69 years (median 39 years) diagnosed with an unroofed CS but without a persistent left superior vena cava (LSVC) underwent transcatheter treatment. Computerized tomography (CT) was performed in eight patients. Transesophageal echocardiography (TEE) was used to monitor the procedure. RESULTS: The mean Qp/Qs ratio was 2.4 ± 1 and mean systolic pulmonary artery pressure was 35 ± 19 mm Hg. An Amplatzer septal occluder was deployed in all nine patients. The device was implanted in the defect in one patient and at the CS ostium in the other eight patients. The median device size used was 22 mm (16-28 mm). The left disc herniated into the CS in the single patient in whom the device was implanted within the defect. All patients were available for the 3-month follow-up. None had a residual shunt on the 3-month follow-up echocardiography. One patient died of a stroke 4.5 months after the procedure. At mean follow-up of 42.6 ± 18.3 months, symptomatic improvement was documented in the remaining eight patients. All eight patients had an O2 saturation above 96%. CONCLUSION: Transcatheter treatment for unroofed CS without persistent LSVC using Amplatzer septal occluder is safe and feasible.

Coronary Diameters in Taiwanese Children Younger than 6 Years Old: Z-Score Regression Equations Derived from Body Surface Area.

BACKGROUND: The measurements of coronary diameters, usually obtained by 2-dimentsional echocardiography, play important roles oin the management and follow-up of Kawasaki disease (KD). However, in Taiwan, domestic normgrams and a Z-score calculator for coronary artery diameters are still not available. METHODS: Echocardiography was performed on 412 healthy children younger than 6 years of age. The appropriate exponential regression model was fitted to correspond with body surface area (BSA). The computed Z-scores of all subjects were also tested for normal distribution. RESULTS: Using the model ln (measurement) = ß1 + ß2 × ln (BSA), the adjusted R(2) values were 0.611 and 0.484 for the models of the left main coronary artery (LMCA) and the right (RCA), respectively. Analysis of computed Z-score distribution showed acceptable goodness of fit for a normal distribution [p = 0.90 (LMCA); p = 0.17 (RCA)]. CONCLUSIONS: We have established reference ranges for the coronary artery diameters in Taiwanese children younger than 6 years of age. The regression equations and Z-score calculators for the LMCA and RCA provide an objective determination of coronary dilatation in a large population, which is important for the care and medical management of KD patients in Taiwan. KEY WORDS: Coronary diameter; Kawasaki disease; Taiwan; Z-score.

Isolated Pulmonary Arteriovenous Fistula in a Patient with Recurrent Stroke.

UNLABELLED: Paradoxical embolism is a risk factor for cryptogenic stroke in young adults, and can be primarily attributed to the presence of patent foramen ovale (PFO). Although contrast echocardiography may facilitate diagnosis, it would be difficult to distinguish a PFO from a pulmonary arteriovenous fistula (PAVF), which could also result in paradoxical embolism. We report a 46-year-old woman with recurrent ischemic stroke, who was scheduled for PFO closure because of a right-to-left shunt detected by contrast echocardiography. The diagnosis turned out to be a PAVF confirmed by angiography. Thereafter, coil embolization of this PAVF was performed successfully. KEY WORDS: Embolic stroke; Patent foramen ovale; Pulmonary arteriovenous fistula; Right-to-left shunt.

Exercise Capacity and Ventricular Remodeling After Transcatheter Ventricular Septal Defect Closure in Asymptomatic or Minimally Symptomatic Adolescents and Adults.

BACKGROUND: Transcatheter ventricular septal defect (VSD) closure is a safe and efficacious alternative to surgery. However, its benefits in asymptomatic or minimally symptomatic patients remain unknown. METHODS: Sixty patients with VSD aged 12 to 60 years underwent cardiopulmonary exercise test and echocardiography 1 day before transcatheter VSD closure and 6 months after intervention (closure group). Thirty patients who did not receive the intervention underwent the same evaluations over 6 months (observation group). RESULTS: No significant change in exercise function was observed after VSD closure, except for increased peak oxygen (O2) pulse (absolute increase: 0.4±1.4 mL/beat). Left ventricular end-diastolic dimension and mitral peak early filling velocity-to-early diastolic annular velocity ratio decreased (absolute decrease: 0.3±0.6 cm and 0.7±1.9, respectively). Despite unchanged overall peak O2 consumption, 33% of closure group patients exhibited clinically significant improvements in peak O2 consumption (>10% increase relative to baseline). This was not related to the pulmonary flow-to-systemic flow ratio or baseline exercise capacity. By contrast, all exercise function parameters deteriorated significantly in the observation group. Subgroup analysis revealed that patients with a baseline left ventricular end-diastolic dimension Z score of >2 exhibited a significantly greater improvement in peak O2 consumption, peak O2 pulse, and oxygen uptake efficiency slope than did the observation group. CONCLUSIONS: Compared with conservative management, transcatheter VSD closure prevents deterioration in exercise capacity and promotes left ventricular reverse remodeling in asymptomatic or minimally symptomatic patients. These benefits are most prominent in patients whose left ventricular end-diastolic dimension Z score before intervention is >2, irrespective of baseline peak O2 consumption and pulmonary flow/systemic flow ratio. Registration: URL: http://www.clinicaltrials.gov. Unique identifier: NCT03127748.

Diagnosis of inferior sinus venosus atrial septal defects using transthoracic three-dimensional echocardiography.

The authors report the cases of 2 patients with symptoms and signs related to severe pulmonary hypertension. Two-dimensional echocardiography demonstrated defects in the posterior portion of the atrial septum. Transthoracic three-dimensional echocardiography clearly showed inferior sinus venosus atrial septal defects and their relationships with the inferior vena cava, the pulmonary vein, and the muscular border of the fossa ovalis. These 2 cases not only elucidate the potential value of transthoracic three-dimensional echocardiography in delineating the structural characteristics of unusual interatrial shunting but also remind clinicians to be aware of this potentially treatable cardiac defect during workup for pulmonary hypertension.

Clinical implications of major aortopulmonary collateral arteries in patients with right isomerism.

BACKGROUND: The presence of major aortopulmonary collateral arteries (MAPCA) in patients with right isomerism may alter the prognosis, which is still unclear. METHODS: From 1994 to 2003, 138 out of 155 patients (89%) with right isomerism had pulmonary stenosis or pulmonary atresia and constituted the study population. RESULTS: Two groups of patients were identified, one with MAPCA and the other without. The MAPCA originating from descending thoracic aorta or aortic arch was noted in 9 patients (6.5%), with a median number of MAPCA being 3 (range, 2 to 4). Patients with MAPCA commonly had congestive heart failure (8/9, 89%) and frequent lower airway infection (6/9, 67%). In comparison with patients with MAPCA and without, the intrapericardial pulmonary artery was more frequently absent (2 of 9 vs 1 of 129, p < 0.001), and the pulmonary arterial size was smaller (McGoon ratio <1.5, 8 of 9 vs 57 of 129, p = 0.009) in the MAPCA group. The mean pulmonary arterial pressure measured was often higher than the suggested limit for Fontan circulation. Major aortopulmonary collateral arteries were addressed at a median of 6 months old (2 to 19 months old) in 5 patients. Although surgical mortality was not different for first-stage palliation and Glenn shunt in both groups, total cavopulmonary connection could only be performed in one patient with MAPCA with poor results. However, the 5-year survival of patients with and without MAPCA was not statistically different (74.1% vs 55.2%, p = 0.19). CONCLUSIONS: The presence of MAPCA was uncommon but still noted in patients with right isomerism. It may cause heart failure and a less favorable pulmonary vasculature for a complete Fontan-type operation.

Clinical implication of hiatal hernia in patients with right isomerism.

OBJECTIVE: Despite a reported association between hiatal hernia in patients with heterotaxy syndrome, the clinical significance has remained unclear. Based on large patient cohorts, this study specifically aimed at defining the implication of hiatal hernia in patients with right isomerism. METHODS: From 1994 to 2002, 143 patients were identified as having right isomerism. Among them, 119 received ultrafast computed tomography (CT) to determine the presence of hiatal hernia as well as any cardiovascular anomalies. RESULTS: Hiatal hernia was found in 17 patients (seven females and 10 males, 14.3%). The upper gastrointestinal (GI) series in six patients confirmed the diagnosis of hiatal hernia in all and revealed severe gastro-oesophageal reflux in four. The most common symptom of hiatal hernia was vomiting (47%), followed by recurrent bronchiolitis or pneumonia (41.2%) and upper gastrointestinal bleeding (11.8%). Three patients with hiatal hernia underwent fundoplication for medically refractory vomiting. Seven patients were found to have midgut malrotation (5.9%), and four of these had both hiatal hernia and malrotation. Pulmonary atresia was closely associated with the presence of hiatal hernia (p=0.02). One patient with hiatal hernia died suddenly at 6 mo. However, overall mortality was similar between those patients with hiatal hernia and those without. CONCLUSIONS: The incidence of hiatal hernia was considerably high in patients with right isomerism, especially in those with pulmonary atresia, and it is this that may have led to vomiting and recurrent airway infections. While an association between sudden death and hiatal hernia may well exist, this does require further clarification.